CD371-positive pediatric B-cell acute lymphoblastic leukemia : propensity to lineage switch and slow early response to treatment
© 2024 American Society of Hematology. Published by Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies..
RUNX1 frequency, immunophenotypic immaturity (all P < .001), and strong expression of CD34 and of CD45 (P < .05). During induction therapy, CD371pos BCP-ALLs showed a transient myelomonocytic switch (mm-SW: up to 65.4% of samples at day 15) and an inferior response to chemotherapy (slow early response, P < .001). However, the 5-year event-free survival was 88.3%. Among 420 patients from the validation cohort, 27 of 28 (96.4%) cases positive for DUX4-fusions were CD371pos. In conclusion, in the largest pediatric cohort, CD371 is the most sensitive marker of transient mm-SW, whose recognition is essential for proper MFC MRD assessment. CD371pos is associated to poor early treatment response, although a good outcome can be reached after MRD-based ALL-related therapies.
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E-Artikel |
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Erscheinungsjahr: |
2024 |
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Erschienen: |
2024 |
Enthalten in: |
Zur Gesamtaufnahme - volume:143 |
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Enthalten in: |
Blood - 143(2024), 17 vom: 25. Apr., Seite 1738-1751 |
Sprache: |
Englisch |
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Journal Article |
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Date Completed 25.04.2024 Date Revised 25.04.2024 published: Print Citation Status MEDLINE |
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doi: |
10.1182/blood.2023021952 |
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PPN (Katalog-ID): |
NLM367059428 |
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100 | 1 | |a Buldini, Barbara |e verfasserin |4 aut | |
245 | 1 | 0 | |a CD371-positive pediatric B-cell acute lymphoblastic leukemia |b propensity to lineage switch and slow early response to treatment |
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500 | |a Citation Status MEDLINE | ||
520 | |a © 2024 American Society of Hematology. Published by Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies. | ||
520 | |a ABSTRACT: In the effort to improve immunophenotyping and minimal residual disease (MRD) assessment in acute lymphoblastic leukemia (ALL), the international Berlin-Frankfurt-Münster (iBFM) Flow Network introduced the myelomonocytic marker CD371 for a large prospective characterization with a long follow-up. In the present study, we aimed to investigate the clinical and biological features of CD371-positive (CD371pos) pediatric B-cell precursor ALL (BCP-ALL). From June 2014 to February 2017, 1812 pediatric patients with newly diagnosed BCP-ALLs enrolled in trial AIEOP-BFM ALL 2009 were evaluated as part of either a screening (n = 843, Italian centers) or validation cohort (n = 969, other iBFM centers). Laboratory assessment at diagnosis consisted of morphological, immunophenotypic, and genetic analysis. Response assessment relied on morphology, multiparametric flow cytometry (MFC), and polymerase chain reaction (PCR)-MRD. At diagnosis, 160 of 1812 (8.8%) BCP-ALLs were CD371pos. This correlated with older age, lower ETV6::RUNX1 frequency, immunophenotypic immaturity (all P < .001), and strong expression of CD34 and of CD45 (P < .05). During induction therapy, CD371pos BCP-ALLs showed a transient myelomonocytic switch (mm-SW: up to 65.4% of samples at day 15) and an inferior response to chemotherapy (slow early response, P < .001). However, the 5-year event-free survival was 88.3%. Among 420 patients from the validation cohort, 27 of 28 (96.4%) cases positive for DUX4-fusions were CD371pos. In conclusion, in the largest pediatric cohort, CD371 is the most sensitive marker of transient mm-SW, whose recognition is essential for proper MFC MRD assessment. CD371pos is associated to poor early treatment response, although a good outcome can be reached after MRD-based ALL-related therapies | ||
650 | 4 | |a Journal Article | |
650 | 4 | |a Research Support, Non-U.S. Gov't | |
650 | 4 | |a Multicenter Study | |
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700 | 1 | |a Varotto, Elena |e verfasserin |4 aut | |
700 | 1 | |a Maurer-Granofszky, Margarita |e verfasserin |4 aut | |
700 | 1 | |a Gaipa, Giuseppe |e verfasserin |4 aut | |
700 | 1 | |a Schumich, Angela |e verfasserin |4 aut | |
700 | 1 | |a Brüggemann, Monika |e verfasserin |4 aut | |
700 | 1 | |a Mejstrikova, Ester |e verfasserin |4 aut | |
700 | 1 | |a Cazzaniga, Giovanni |e verfasserin |4 aut | |
700 | 1 | |a Hrusak, Ondrej |e verfasserin |4 aut | |
700 | 1 | |a Szczepanowski, Monika |e verfasserin |4 aut | |
700 | 1 | |a Scarparo, Pamela |e verfasserin |4 aut | |
700 | 1 | |a Zimmermann, Martin |e verfasserin |4 aut | |
700 | 1 | |a Strehl, Sabine |e verfasserin |4 aut | |
700 | 1 | |a Schinnerl, Dagmar |e verfasserin |4 aut | |
700 | 1 | |a Zaliova, Marketa |e verfasserin |4 aut | |
700 | 1 | |a Karawajew, Leonid |e verfasserin |4 aut | |
700 | 1 | |a Bourquin, Jean-Pierre |e verfasserin |4 aut | |
700 | 1 | |a Feuerstein, Tamar |e verfasserin |4 aut | |
700 | 1 | |a Cario, Gunnar |e verfasserin |4 aut | |
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700 | 1 | |a Möricke, Anja |e verfasserin |4 aut | |
700 | 1 | |a Biffi, Alessandra |e verfasserin |4 aut | |
700 | 1 | |a Parasole, Rosanna |e verfasserin |4 aut | |
700 | 1 | |a Fagioli, Franca |e verfasserin |4 aut | |
700 | 1 | |a Valsecchi, Maria Grazia |e verfasserin |4 aut | |
700 | 1 | |a Biondi, Andrea |e verfasserin |4 aut | |
700 | 1 | |a Locatelli, Franco |e verfasserin |4 aut | |
700 | 1 | |a Attarbaschi, Andishe |e verfasserin |4 aut | |
700 | 1 | |a Schrappe, Martin |e verfasserin |4 aut | |
700 | 1 | |a Conter, Valentino |e verfasserin |4 aut | |
700 | 1 | |a Basso, Giuseppe |e verfasserin |4 aut | |
700 | 1 | |a Dworzak, Michael N |e verfasserin |4 aut | |
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